Supportive measures in intensive care units, treatments for specific aetiologies and all the advances made in liver transplantation, including surgical techniques, immunosuppression and post-transplant care, have increased survival from 60% to 80%. 7,8 Although the actual incidence of paediatric ALF is unknown, it is estimated that it is the reason for 10% to 15% of all liver transplants performed in children. 4–6 However, there is a considerable number of cases (between 40% and 50%) where the aetiology cannot be determined. Viral and autoimmune diseases and poisoning are the most frequent causes in older age groups. In Europe and North America, metabolic disorders and viral infections are the most frequent causes of ALF in newborns. The aetiology of ALF varies by age group and geographical area. This disease carries a high mortality risk or requires liver transplantation in 70% of cases. Paediatric acute liver failure (ALF) is a multisystem disorder that gives rise to severe liver failure within days or weeks, manifests with or without encephalopathy, and occurs in children with no pre-existing chronic liver disease. El valor del PELD puede ayudar a establecer el momento óptimo para inclusión en lista de TH sin embargo son necesarios estudios a mayor escala. Los pacientes con FHA que presentaron un PELD elevado junto con encefalopatía tuvieron peor evolución. La supervivencia de FHA sin necesidad de TH fue más probable en aquellos con valores de PELD bajos y que no desarrollaron encefalopatía, con un RR de 0,326. Un punto de corte de 27 en el PELD según la curva ROC mostró una sensibilidad del 86% y una especificidad del 85% de presentar evolución desfavorable (ABC: 0,90 p < 0,001). Los pacientes con cifras elevadas de bilirrubina, INR o que desarrollaron encefalopatía tuvieron más probabilidades de presentar una evolución tórpida, obteniéndose una OR para el INR de 1,93. Los pacientes que requirieron TH fueron el 42,8%, y el 16,3% fallecieron. Las etiologías más frecuentes fueron la indeterminada (36,7%) y la metabólica (26,5%). Pacientes y métodosĮstudio retrospectivo de pacientes con FHA en nuestro centro de 2000 a 2013 según criterios del grupo de trabajo de FHA. ObjetivosĮvaluar las características etiológicas y la evolución de niños con FHA en un centro con trasplante hepático (TH) infantil e investigar la validez del PELD como indicador pronóstico. La puntuación Pediatric End-stage Liver Disease (PELD) es un predictor de mortalidad en hepatopatías crónicas, siendo la experiencia en FHA limitada. The PELD score could be a useful tool to establish the optimum time for inclusion in the transplant list, however further studies are still needed.Įl fallo hepático agudo (FHA) es una enfermedad multisistémica con afectación severa de la función hepática de aparición brusca. ConclusionsĪLF patients with a high PELD score and the presence of encephalopathy had worse outcomes. The survival of patients with ALF without transplantation seems more likely in those who have low values of PELD and absence of encephalopathy, with a RR of 0.326. A cut-off of 27 in the PELD score according to the ROC curve showed a sensitivity of 86% and a specificity of 85%, predicting a worse outcome (AUC: 0.90 P <. Patients with higher levels of bilirubin, INR, or encephalopathy were more likely to require a liver transplant, yielding an OR for INR 1.93. Liver transplant (LT) was required by 42.8%, and there were 16.3% deaths. The most frequent aetiologies were: indeterminate (36.7%) and metabolic (26.5%). The study included 49 patients with an age range 0–14 years. ![]() Patients and methodsĪ retrospective study was conducted on patients diagnosed with ALF in our hospital from 2000 to 2013 using the criteria of the Paediatric ALF Study Group. To evaluate the aetiology and outcomes of children with ALF in a Children's Liver Transplant Centre, and to investigate the validity of PELD as a prognostic indicator. The Paediatric End-stage Liver Disease (PELD) score is used as a predictor of mortality in chronic liver disease, however experience is limited in ALF. Acute liver failure (ALF) is a multisystem disease with severe impairment of liver function of acute onset.
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